Plasma exchange for anti GAD associated non paraneoplastic limbic encephalitis.
Limbic encephalitis (LE) is a neurological syndrome usually presenting in a paraneoplastic form. Recently many cases were reported with no concurring neoplasia, presenting with specific antibodies for voltage-gated potassium channel or for neuronal membrane antigens. Anti-glutamic acid decarboxylase (GAD) antibodies act against GABAergic receptors of the central nervous system. These antibodies were found in coeliac disease serum and in neurologic patients. We are reporting a case of a 21-year-old coeliac woman manifesting complex multiple-daily partial drug-resistant seizures for 7 years. The diagnosis was of a non paraneoplastic limbic encephalitis, unresponsive to high dose cortisone and IGIV infusion. The use of therapeutic plasma exchange (TPE) has resulted in an improvement in symptoms with quite a long disease-free period of time. When the frequency of the procedures was decreased, seizures appeared again and, after suspension of TPE, the clinical status worsened. The role of TPE in the treatment of LE still has to be defined.
Mazzi
Gustavo
G
Servizio di Immunoematologia e Medicina Trasfusionale, Azienda Ospedaliera. "Santa Maria degli Angeli" Via Montereale 24, 33170 Pordenone, Italy. gustavo.mazzi@aopn.fvg.it
Roia
Dina De
DD
Cruciatti
Barbara
B
Matà
Sabrina
S
Catapano
Raffaele
R
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D002446:Celiac Disease / Q000097:blood / Q000276:immunology / Q000628:therapy; D003348:Cortisone / Q000008:administration & dosage; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage; D007155:Immunologic Factors / Q000008:administration & dosage; D020363:Limbic Encephalitis / Q000097:blood / Q000276:immunology / Q000628:therapy*; D010951:Plasma Exchange
D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D005968:Glutamate Decarboxylase; D003348:Cortisone